William J. Power

Keratoprosthesis: preoperative prognostic categories.

Purpose. Recent advances aimed at preventing and treating complications after keratoprosthesis surgery have improved prognosis, but it has been suspected that various preoperative diagnoses may carry substantially different postoperative outcomes. This article attempts to clarify the ranking of prognostic categories for patients undergoing keratoprosthesis surgery. Methods. A retrospective review of the outcome in a recent series of 63 patient eyes operated at the Massachusetts Eye and Ear Infirmary between 1990 and 1997 and followed up for a minimum of 21 months. Anatomic retention of the device and the loss of vision caused by complications were recorded. The patients were divided into four categories according to preoperative cause. Results. Anatomically, one keratoprosthesis extruded spontaneously. Another 10 were permanently removed because of complications. Of the 63 eyes, 10 never achieved a visual acuity of at least 20/200 vision because of preexisting retinal or optic nerve damage. The remaining 53 had a visual acuity of 20/200 to 20/20 as follows: Stevens–Johnson syndrome (n = 7), after 2 years: 33%, after 5 years: 0%; chemical burn (n = 17), after 2 years: 64%, after 5 years: 25%; ocular cicatricial pemphigoid (n = 20), after 2 years: 72%, after 5 years: 43%; graft failure in noncicatrizing conditions (dystrophies, degenerations, or bacterial or viral infections) when a repeat graft was expected to have a poor prognosis (n = 19), after 2 years: 83%, after 5 years: 68%. The difference in outcome between the Stevens–Johnson syndrome outcome group and the graft failure group or the ocular cicatricial pemphigoid group was statistically significant. In the group of 53 eyes, visual acuity was restored to 20/200 to 20/20 for a cumulative total of 138 years. Conclusion. Outcome of the keratoprosthesis surgery varied markedly with preoperative diagnosis. Most favorable was graft failures in noncicatrizing conditions, whereas Stevens–Johnson syndrome was the worst. Ocular cicatricial pemphigoid and chemical burns occupied a middle ground. The difference between the groups seemed to correlate with the degree of past preoperative inflammation.

Analysis of the Acute Ophthalmic Manifestations of the Erythema Multiforme/Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Disease Spectrum

PURPOSE To evaluate the epidemiology, possible etiologic factors, complications encountered, and treatment administered to a group of patients with ocular involvement in the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum who were seen at two large tertiary referral centers over a 34-year period. METHODS Hospital records from 1960 to 1994 at the Massachusetts General Hospital and Shriners Hospital for Crippled Children were reviewed for patients with erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis. Only patients fulfilling specific clinical diagnostic criteria and those who received a diagnosis by a dermatologist were included in the review. RESULTS A total of 366 patients with erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis were identified. Drugs were the most commonly identified etiologic factor in all three conditions: sulfonamides were the most frequently identified agents. Eighty-nine patients (24%) had ocular manifestations at the time of their acute hospital stay. Ocular involvement was seen in 9% of patients with erythema multiforme, in 69% with Stevens-Johnson syndrome, and in 50% with toxic epidermal necrolysis. The ocular problems were more severe in patients with both Stevens-Johnson syndrome and toxic epidermal necrolysis. There was no significant difference between the number of patients who were treated with systemic steroids and those who were not (P = 0.42). CONCLUSIONS The erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum remains an important cause of severe visual loss in a significant number of patients. Systemic steroids used during the acute phase of the disease appear to have no effect on the development of ocular manifestations. Studies on the acute immunopathogenic mechanisms occurring in these disease are warranted if more effective therapies are to be found.

Changing patterns in uveitis of childhood.

BACKGROUND Although uveitis is relatively uncommon in children, its diagnosis and management present a distinct clinical challenge for the physician. An improved knowledge of disease patterns and associated morbidity will help in the care of children with uveitis. METHODS The authors reviewed the records of 130 patients with onset of uveitis at 16 years of age or younger. The etiology of uveitis, complications encountered, treatment administered, and visual results were analyzed. RESULTS Uveitis associated with juvenile rheumatoid arthritis (JRA) was the largest group (41.5%) followed by idiopathic uveitis (21.5%) and pars planitis (15.3%). Twenty-six percent of the eyes had less than 20/200 visual acuity at the time of first referral. Patients with JRA had the highest rate of complications: cataract (71%), glaucoma (30%), band keratopathy (66%), and hypotony (19%). The most frequent complication of pars planitis was maculopathy (55%). Final visual acuity was less than 20/200 in 26% of eyes with JRA, 10.5% with pars planitis, and 14% with idiopathic uveitis. CONCLUSION Uveitis beginning in childhood is a serious disease associated with sight-threatening complications. Juvenile rheumatoid arthritis-associated uveitis remains a leading cause of ocular morbidity in patients with childhood uveitis. Increased awareness by pediatricians, rheumatologists, and ophthalmologists of the seriousness of ocular complications of uveitis in childhood may lead to earlier diagnosis and more effective treatment regimens in the future.

Outcomes in anterior uveitis associated with the HLA-B27 haplotype

OBJECTIVE This study aimed to test the hypothesis that patients presenting with anterior uveitis who are HLA-B27 positive, either with or without associated systemic disease, have a less-favorable outcome than do patients with idiopathic anterior uveitis who are HLA-B27 negative. DESIGN Retrospective case-controlled series. PARTICIPANTS Ninety-seven patients who were HLA-B27 positive with no systemic disease, 94 patients who were HLA-B27 positive with systemic disease, and 72 patients who were HLA-B27 negative who presented with anterior uveitis were studied. MAIN OUTCOME MEASURES Ocular complications (e.g., secondary glaucoma, cataract formation, pupillary synechiae, vitritis, cystoid macular edema, and optic disc edema), medical and surgical treatment, number of recurrent attacks, and final visual acuity were recorded for all patients. RESULTS The patients who were HLA-B27 positive, either with or without systemic disease, experienced a greater number of complications than did the patients who were HLA-B27 negative. Periocular corticosteroids, systemic corticosteroids, and systemic immunosuppressive chemotherapy were required in a far greater number of HLA-B27-positive patients than in HLA-B27-negative patients (60% vs. 11%, 53% vs. 7%, and 18% vs. 1%, respectively; P < 0.001). The percentage of legally blind eyes was significantly greater in the HLA-B27-positive group, both with and without systemic disease, when compared with the HLA-B27-negative group (11% vs. 2%; P < 0.005). CONCLUSIONS The prognosis of anterior uveitis associated with the HLA-B27 haplotype, either with or without associated systemic disease, is less favorable when compared with that of HLA-B27-negative patients with idiopathic anterior uveitis.

Secondary Glaucoma in Patients with Uveitis

Purpose: To evaluate the prevalence of secondary glaucoma (SG), clinical forms of uveitis more frequently associated with glaucoma, and describe the treatment and complications encountered in a cohort of patients with glaucoma and uveitis during a 10-year period. Methods: The hospital records of patients with uveitis referred to the Immunology Service of the Massachusetts Eye and Ear Infirmary for a decade were reviewed for cases of SG. Results: One hundred and twenty of the 1,254 patients (9.6%) with uveitis developed SG. SG was more frequent in anterior uveitis (67%) but was also associated with posterior uveitis (13%) and pars planitis (4%). Herpetic keratouveitis (22%), Fuchs’ iridocyclitis (19%), juvenile rheumatoid arthritis-associated iridocyclitis (16%), syphilis (14%), and sarcoidosis (12%) were the leading types of uveitis associated with SG. Despite aggressive medical and surgical therapy, SG was associated with progressive visual field loss and optic nerve damage in 39 patients (33%). Conclusion: SG is an underappreciated, vision-threatening complication in patients with uveitis. Increased vigilance for emergence of this complicating problem during the care of patients with uveitis is warranted, and medical and surgical treatment for reducing IOP should be especially aggressive in these patients. We hypothesize that earlier, more aggressive treatment of uveitis will reduce the presence of glaucoma as an additional vision-robbing complication of uveitis.

Long-term follow-up of patients with atopic keratoconjunctivitis.

OBJECTIVE This study aimed to review the presenting features, treatment administered to, histopathologic findings, and complications encountered in a cohort of patients with atopic keratoconjunctivitis. DESIGN The study design was a retrospective cohort series. PARTICIPANTS The medical records of 20 patients with atopic keratoconjunctivitis and a minimum follow-up of 3 years were reviewed. MAIN OUTCOME MEASURES Conjunctival and corneal complications, visual acuity before and after surgery, and histopathologic features on conjunctival biopsy were measured. RESULTS Significant keratopathy developed in 70% of patients, corneal neovascularization in 60%, fornix foreshortening in 25%, and symblepharon in 20% during the course of their disease. Eleven patients (12 eyes) required penetrating keratoplasty (3 for tectonic purposes and 8 for visual rehabilitation). Vision improved by four or more lines of Snellen acuity in four eyes, improved by two lines in two eyes, remained the same in five eyes, and worsened by two lines in one eye after keratoplasty. Cataract surgery was performed in seven patients (nine eyes) with vision improving by four or more lines in six patients (eight eyes). CONCLUSION Atopic keratoconjunctivitis is a potentially blinding disease that may result in a poor visual outcome as a result of corneal complications. Elective surgical intervention may be of benefit and can be considered in those patients whose inflammation is well controlled.

Effect of topical cyclosporin A on conjunctival T cells in patients with secondary Sjögren's syndrome.

The effect of topical cyclosporin A on conjunctival T cells was studied in nine patients with secondary Sjögrens disease. Patients had conjunctival biopsies performed before and after a 6-week course of topical cyclosporin. Epithelium and substantia propria in the Sjögrens patients before treatment showed significantly more CD4 + cells than specimens taken from nine age- and sex-matched controls. Following treatment with topical cyclosporin, there was a significant reduction in the number of CD4 + cells in both the conjunctival epithelium and substantia propria. Despite the fact that the treatment resulted in immunopathological improvement, the clinical benefit was not as favorable. Our results suggest that topical cyclosporin may have a local immunosuppressive effect on the conjunctiva in patients with Sjögrens disease.

A Prospective Evaluation of Alcohol-assisted versus Mechanical Epithelial Removal Before Photorefractive Keratectomy

OBJECTIVE The purpose of the study is to compare alcohol-assisted versus mechanical debridement of the corneal epithelium before photorefractive keratectomy (PRK) for low-to-moderate myopia. DESIGN A prospective study was performed on a group of consecutive patients operated on at the Massachusetts Eye and Ear Infirmary from February to April 1996 and followed for 6 months. PARTICIPANTS Eighty patients (eyes) were divided in 2 groups: 40 alcohol and 40 mechanical. INTERVENTION The patients underwent PRK for myopia (-1.5 to -7.5 diopters) with a Summit Apex excimer laser. The corneal epithelium was removed either with 20% ethanol or with a scalpel blade. MAIN OUTCOME MEASURES The two groups were compared for epithelial removal time, epithelial defect size at the end of surgery, and rate of re-epithelialization. Uncorrected visual acuity (UCVA), refractive outcome, best-corrected visual acuity (BCVA), and subjective haze were measured at 4 days and at 1, 3, and 6 months. In an additional short-term study, 40 patients (20 alcohol, 20 mechanical) had intraoperative pachymetry performed. RESULTS Alcohol-assisted de-epithelialization was faster than mechanical debridement (107 [+/-20.6 standard deviation] versus 141 [+/-30.5] seconds [P < 0.0001]) and led to a more circumscribed and reproducible epithelial defect at the end of surgery (87,739 [+/-11,852] versus 103,518 [+/-33,942] square pixels [t test, P = 0.04; f test, P = 0.001]). At 4 days, 95% of the alcohol-treated patients had healed compared with 78% of the mechanically scraped patients (Fishers exact test, P = 0.04). The alcohol group had a better UCVA at 4 days (logarithm of the minimum angle of resolution UCVA 0.36 [+/-0.22] versus 0.51 [+/-0.26]) and at 1 month (0.14 [+/-0.17] versus 0.22 [+/-0.16] [Mann-Whitney U test, P = 0.02 and P = 0.03]) but equalized at 3 months (0.10 [+/-0.14] versus 0.13 [+/-0.16]) and at 6 months (0.11 [+/-0.15] versus 0.14 [+/-0.13] [Mann-Whitney U test, P = 0.23 and P = 0.34]). There was a trend toward less subjective haze in the alcohol-treated patients over the course of the study (area under the curve, 71.9 [+/-35.3] versus 87.9 [+/-33.8] [Mann-Whitney U test, P = 0.07]). The difference from target was equivalent in both groups at 6 months (-0.22 [+/-0.58] diopter in the alcohol group and -0.43 [+/-0.52] diopter in the mechanical group [t test, P = 0.14; f test, P = 0.57]). There were no differences in intraoperative pachymetry, corneal uniformity index as calculated from the corneal topography, and loss of BCVA between the two groups. CONCLUSIONS Twenty percent ethanol is a simple, safe, and effective alternative to mechanical scraping before PRK and appears to be associated with a quicker visual rehabilitation.

Increasing the Diagnostic Yield of Conjunctival Biopsy in Patients with Suspected Ocular Cicatricia1 Pemphigoid

BACKGROUND Ocular cicatricial pemphigoid is a chronic, progressive, autoimmune disease that scars mucous membranes and may lead to blindness. It is of critical importance to be able to make the diagnosis as early as possible to allow early treatment. Conjunctival biopsy facilitates the early diagnosis of this condition. MATERIALS AND METHODS Conjunctival biopsy results of 166 consecutive patients seen over a 7-year period, in whom the diagnosis of ocular cicatricial pemphigoid was considered, were reviewed. RESULTS One hundred twenty-one patients ultimately received a diagnosis of ocular cicatricial pemphigoid. Immunofluorescence studies demonstrated characteristic deposition of immunoreactants at the epithelial basement membrane zone in 63 patients (sensitivity = 52%). When immunofluorescent-negative or inconclusive biopsies were processed further using an immunoperoxidase technique an additional 37 diagnoses were made. This represented an increase in sensitivity from 52% with immunofluorescence only to 83% with the addition of the immunoperoxidase technique. CONCLUSION The routine use of the immunoperoxidase technique in immunofluorescent-negative biopsies, allied with appropriate harvesting and handling of biopsied conjunctiva, should significantly increase the diagnostic yield in patients with clinically suspect ocular cicatricial pemphigoid.

Risk factors, microbiological findings, and clinical outcomes in cases of microbial keratitis admitted to a tertiary referral center in Ireland.

Aim: To identify the risk factors for, and to report the microbiological findings and clinical outcomes of, severe microbial keratitis (MK). Methods: This was a retrospective study of all cases of presumed MK admitted to a tertiary referral center over a 2-year period (September 2001 to August 2003). Data recorded included demographic data, details relating to possible risk factors, results of microbiological studies, clinical findings at presentation, and clinical and visual outcomes. Results: Ninety patients were admitted with a diagnosis of presumed MK during the study period. The mean age of patients was 45 ± 32 years, and the male to female ratio was 47:43 (52.2%:47.7%). Predisposing risk factors for MK included contact lens wear (37; 41.1%), anterior segment disease (19; 21.1%), ocular trauma (13; 14.4%), systemic disease (5; 5.6%), and previous ocular surgery (1; 1.1%). Cultured organisms included gram-negative bacteria (17; 51.5%), gram-positive bacteria (11, 33.3%), acanthamoeba (2; 6.1%), and fungi (1; 3%). Visual acuity improved significantly after treatment [mean best-corrected visual acuity (±standard deviation) at presentation: 0.76 (±0.11); mean best-corrected visual acuity at last follow-up: 0.24 (±0.07); P < 0.001]. Secondary surgical procedures were required in 18 (20%) cases, and these included punctal cautery (1; 1.1%), tissue glue repair of corneal perforation (2; 2.2%), tarsorrhaphy (9; 9.9%), Botulinum toxin-induced ptosis (1; 1.1%), penetrating keratoplasty (3; 3.3%), and evisceration (2; 2.2%). Conclusions: Contact lens wear remains a significant risk factor for severe MK. MK remains a threat to vision and to the eye, but the majority of cases respond to prompt and appropriate antimicrobial therapy.